Congenital bile duct dilation —Possibly an hereditary condition—

A female patient underwent resection of a large choledochal cyst and hepaticojejunostomy when she was 11 years old at Tokyo Medical and Dental University Hospital. Her mother, 48 years old, also had congenital bile duct dilatation (CBD) with cancer. The mother had an anomalous pancreatico-biliary ductal junction. To investigate the possibility of a genetic link, family members of another three consecutive CBD cases were examined for bile duct dilatation and anomalous pancreatico-biliary junction. Endoscopic retrograde choledocho-pancreatography (ERCP) revealed an anomalous pancreatico-biliary ductal junction, without bile duct dilatation in the mother of a 17-year-old CBD patient. Two familial occurrences of CBD had been reported in the Japanese literature. Their combinations were; a father—a daughter and two sisters, respectively. Considering that the sex ratio of CBD is one male to 2.6 females, our results suggest that CBD or anomalous pancreatico-biliary ductal junction may be 1) an X-linked dominant trait or 2) autosomal dominant trait with relatively low penetrance in males. Since anomalous pancreatico-biliary ductal junction does not necessarily develop into bile duct dilatation, more familial investigations on pancreatico-biliary ductal junction and bile duct dilatation are essential to clarify the genetic contribution to CBD.     

Congenital bile duct dilatation--possibly an hereditary condition

A female patient underwent resection of a large choledochal cyst and hepaticojejunostomy when she was 11 years old at Tokyo Medical and Dental University Hospital. Her mother, 48 years old, also had congenital bile duct dilatation (CBD) with cancer. The mother had an anomalous pancreatico-biliary ductal junction. To investigate the possibility of a genetic link, family members of another three consecutive CBD cases were examined for bile duct dilatation and anomalous pancreatico-biliary junction. Endoscopic retrograde choledocho-pancreatography (ERCP) revealed an anomalous pancreatico-biliary ductal junction, without bile duct dilatation in the mother of a 17-year-old CBD patient. Two familial occurrences of CBD had been reported in the Japanese literature. Their combinations were; a father--a daughter and two sisters, respectively. Considering that the sex ratio of CBD is one male to 2.6 females, our results suggest that CBD or anomalous pancreatico-biliary ductal junction may be 1) an X-linked dominant trait or 2) autosomal dominant trait with relatively low penetrance in males. Since anomalous pancreatico-biliary ductal junction does not necessarily develop into bile duct dilatation, more familial investigations on pancreatico-biliary ductal junction and bile duct dilatation are essential to clarify the genetic contribution to CBD.     

Mechanism of the bile duct dilatation in congenital dilatation of the common bile duct

Radiographic studies including operative cholangiography, endoscopic retrograde cholangiopancreaticography and percutaneous transhepatic cholangiography were performed in 44 patients with congenital dilatation of the common bile duct. The relation between the maximum transverse diameter of the common bile duct (CBD) as an indicator of the degree of dilatation and various possible factors causing dilatation of CBD was studied. Among these factors, the length of the narrow segment (X) was closely related with the maximum transverse diameter (Y). The relation of these two was expressed by a simple equation; log Y = 0.068X + 1.06 (r = 0.934, p less than 0.001, n = 19) Dilatation of CBD has been successfully produced by narrowing the ductal lumen with a small plastic tube encirculating the distal common bile duct in dogs. Experimental dilatation was quite similar with congenital dilatation. In addition, the relation between the maximum transverse diameter (Y') and the length of the narrow segment (X') was also expressed in the following simple equation; log Y' = 0.060X' + 0.88 (r = 0.972, p less than 0.001, n = 18) The present studies did not support the hypothesis that abnormal junction of the pancreatico-biliary system might be the primary cause of congenital dilatation of CBD. They suggest that the stenosis of the distal CBD in the fetal life may determine dilatation of CBD.     

Choledochal cyst originating from primary sclerosing cholangitis in a child

Choledochal cysts are common in Asian children. Primary sclerosing cholangitis (PSC), which is characterized by inflammation and fibrosis and may lead to bile duct stricture over the intrahepatic or extrahepatic bile duct, is rare in children. Here we report a case of a 10-year-old boy who presented with a choledochal cyst originating from PSC. He had suffered from repeated abdominal pain and cholangitis for 3 years. A type IV choledochal cyst was suspected from the ultrasound and computed tomography image showing a distended gallbladder and dilatation of the bilateral intrahepatic duct at the hepatic hilar area and common bile duct (CBD). During laparotomy, a markedly distended gallbladder was noted and was shown to have no communication with the CBD by intraoperative cholangiogram. Choledochal cysts with extrahepatic and intrahepatic duct dilatation at the hilar area and marked stenosis with nearly total obstruction of the distal CBD were noted. Hepaticojejunostomy was performed. The histopathologic findings demonstrated a typical PSC picture. The patient's postoperative course was uneventful for 8 months after surgery, and he received no medication during a regular follow-up.     

Clinical and experimental studies on the etiology of congenital biliary dilatation (CBD)--with special reference to relationship between elastic fibers in the wall of the bile duct and type of CBD

The purposes of this study are to clarify the correlation between the morphological differences in congenital biliary dilatation (CBD) and elastic fibers in the wall of the bile duct. Using forty-three patients with CBD and fifty-seven autopsy cases in which history of biliary disease had not demonstrated, histopathological studies have been performed. In addition, experimental models of anomalous choledocho-pancreatico ductal junction were created in twenty-two young sheep and nine neonatal lambs, and the differences in morphological change were studied in two groups. The results are as follows. 1) Elastic fibers were not seen in the wall of the common bile duct of neonate or infant. So it suggests that the ductal wall was extremely weak in this period and increased intraluminal pressure of the biliary system, induced by distal obstruction, caused the cystic dilatation easily. 2) Fusiform dilatation of the extrahepatic bile duct was produced in all experimental models. The reason why cystic dilatation was not caused in these models is that elastic fibers were abundant in just ductal wall of neonatal lambs. From these results, aging factors may play an important role in the formation of CBD.     

内镜下乳头气囊扩张术治疗胆囊切除术后胆总管结石

目的　探讨经内镜乳头气囊扩张术 (EPBD)治疗胆囊切除术后胆总管结石的安全性和疗效。方法　对胆囊切除术后出现黄疸或胆管炎的 31例患者 ,利用EPBD结合取石篮、取石球囊或总攻方法 ,使梗阻于胆总管下段的结石排入肠道。结果　 31例患者全部顺利实施EPBD ,共排除结石37颗 ,4例行 2次扩张术 ,术后患者均痊愈出院 ,复查B超无结石残存或胆管扩张。结论　经内镜治疗胆囊切除术后胆总管结石 ,安全、无创、有效 ,有望成为治疗LC术后胆总管结石的首选方法     

Association of extrahepatic bile duct duplication with pancreaticobiliary maljunction and congenital biliary dilatation in children: a case report and literature review

We herein report a case of cystic-type congenital biliary dilatation (CBD) in whom an extremely rare anomalous duplication of the common bile duct and pancreaticobiliary maljunction were diagnosed intraoperatively by meticulous surgical manipulations via conventional open surgery. By performing a dissection at the outer epicholedochal layer of the cyst, a thin cord-like structure shown to be the distal part of the common bile duct was identified. A further exploration revealed that the most distal (extra- and intrapancreatic) part of the common bile duct was duplicated, and each branch of the duct was connected to the main and accessory pancreatic ducts. The experience with our case and a literature review showed that extrahepatic bile duct duplication is generally associated with pancreaticobiliary maljunction and CBD. We conclude that an extremely careful exploration with delicate and meticulous surgical manipulation is essential to identify these morphological anomalies and prevent intraoperative and postoperative complications of CBD, such as pancreatic duct injury or pancreatitis.     

Pancreatobiliary fistula associated with an intraductal papillary-mucinous pancreatic neoplasm manifesting as obstructive jaundice: Report of a case

We report a pancreatobiliary fistula caused by an intraductal papillary-mucinous pancreatic neoplasm (IPMN), manifesting as obstructive jaundice. Computed tomography showed dilatation of the bile duct and main pancreatic duct, with multiple cystic masses in the head of the pancreas. Endoscopic retrograde pancreatocholangiography showed a patulous papilla with mucin secretion. Contrast enhancement outlined amorphous material obstructing the lower part of the common hepatic duct. Pancreatogram and magnetic resonance cholangiopancreatography showed diffuse dilatation of the main pancreatic duct and side branches without communication with the adjacent organs or duct. We performed pancreaticoduodenectomy for IPMN of the pancreatic head and a tumor-like lesion in the lower common bile duct (CBD). Macroscopically, impacted thick mucus protruded into the CBD from the pancreas via a pancreatobiliary fistula. Histologic examination revealed a pancreatobiliary fistula caused by intraductal papillary-mucinous carcinoma of the pancreas with mucin hypersecretion, an adenoma without interstitial infiltration, and isolated implantation of an IPMN in the bile duct mucosa around the fistula.     

Congenital biliary dilatation may consist of 2 disease entities

Background/Purpose

This study aims to establish the possible mechanisms of pathogenesis of congenital biliary dilatation and to classify the disease accordingly.

Methods

Radiologic features of congenital biliary dilatation and pancreaticobiliary malunion in 107 affected children were examined and correlated with laboratory results. Relative lengths/diameters were calculated to provide comparison between children of different ages. Intraluminal pressures of common bile duct (CBD) were measured intraoperatively.

Results

The minimal relative diameters of distal CBD negatively correlated with the maximal relative diameters/lengths of dilated CBD, the maximal relative diameters of common hepatic duct, and left/right hepatic ducts. The intraluminal pressure in patients with a stenotic distal CBD (stenotic group) was significantly higher than that in patients with a nonstenotic distal CBD (nonstenotic group). The narrower the distal CBD, the more deranged the liver function. Conversely, serum/bile amylase levels were more elevated in the nonstenotic group. Common channel protein plugs were only found in the nonstenotic group, whereas common hepatic duct strictures, intrahepatic duct dilatations, and calculi were detected more frequently in the stenotic group.

Conclusion

We propose to categorize congenital biliary dilatation into 2 subgroups: (1) cystic type with stenotic distal CBD associated with deranged liver function and common hepatic duct stricture and (2) fusiform type with nonstenotic distal CBD associated with pancreatitis and common channel protein plugs. Different underlying pathologies of each group require different operative strategies.     

经内镜乳头气囊扩张术治疗胆总管结石102例分析

目的分析经内镜气囊扩张治疗胆总管结石的疗效和近期并发症。方法回顾性分析2003年3月至2009年5月经内镜气囊扩张治疗胆总管结石102例的疗效及并发症的防治。结果本组取石成功率为98.0%。术后出现一过性高淀粉酶血症11例,其中急性胰腺炎5例(4.9%),经药物治疗后痊愈。无一例发生胆道感染、出血、穿孔等严重并发症。结论经内镜气囊扩张治疗胆总管结石疗效好,并发症发生率低,是安全、有效的微创治疗措施。     

Right posterior branch draining into intra-pancreatic common duct: the triple duct sign

Simultaneous dilatation of pancreatic duct (PD) and common bile duct (CBD) is often seen on radio imaging in pancreatic head malignancy or chronic pancreatitis. This is called double duct sign. However, dilatation of aberrant bile duct along with PD and CBD resulting in triple duct sign on radio imaging has not been reported in literature till date. We report a case of 45 years old male with surgical jaundice due to pancreatic head mass. Computed tomography (CT) scan revealed three dilated ducts in the head of pancreas. Besides PD and biliary duct, the third duct was pre-operatively thought to be long cystic duct with low insertion in common hepatic duct (CHD). Intra-operative findings revealed an unusually long and dilated aberrant right posterior sectoral duct with low insertion into intra-pancreatic CHD. Patient underwent Whipple’s procedure with two separate biliary anastomosis besides pancreaticojejunostomy. Histopathology of specimen revealed chronic pancreatitis. Retrospective analysis shows that Magnetic Resonance Cholangiopancreatogram (MRCP) should have been done for correct pre-operative delineation of this anatomical anomaly. In conclusion, pre-operative detection of triple duct dilatation on CT scan should be further investigated with MRCP for anatomical variations of the biliary tract. This can guide operative planning and prevent inadvertent biliary injuries.     

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先天性胆管扩张症是指肝内、外胆管单独或联合的先天性发育异常,多发生于女性,常在婴幼儿期发现。随着医学影像检查在临床应用,少数于成年期发现确诊。先天胆管扩张症多发生于胆总管,并呈梭形扩张,最多见者为I型(先天性胆总管囊肿)。扩张的胆管内胆汁淤滞潴留,可并发胆管炎、结石、穿孔,少数囊壁发生恶变。先天性胆总管囊肿于成年期一经确诊,无论症状轻重,如无手术禁忌证均应手术治疗。目前主张,除非有特殊的适应证,不宜采取内引流或外引流术。彻底切除囊肿,重建肝外胆道是根治先天性胆总管囊肿的标准术式。     

An unusual cause of delayed presentation of laparoscopic common bile duct injury

We describe an unusual case of a laparoscopic common bile duct (CBD) injury that presented with cholangitis 2 years after an apparently uneventful laparoscopic cholecystectomy. Preoperative ultrasound and endoscopic retrograde cholangiography suggested choledocholithiasis, showing proximal and intrahepatic duct dilatation with an inability to relieve the obstruction. At surgery, a lateral injury of the CBD wall with partial wall loss was found, adherent to an amorphous pigmented mass with the appearance of a knitted fabric causing CBD obstruction. The CBD was successfully reconstructed with a Roux-en-Y end-to-side hepaticojejunostomy, where the end of the Roux loop was anastomosed to the lateral CBD defect.     

Invasive cholangiocarcinoma identified in congenital biliary dilatation in a 3-year-old boy

We herein report a 3-year-old male demonstrating invasive cholangiocarcinoma (CC) associated with congenital biliary dilatation (CBD). A 3-year-old Japanese boy was admitted to our hospital with abdominal pain and vomiting. Computed tomography and magnetic resonance imaging demonstrated a dilated extrahepatic bile duct. A diagnosis of Todani's type 1a CBD was made.Intraoperative cholangiography demonstrated the presence of pancreaticobiliary maljunction but could not reveal any tumor lesion in the bile duct. The excision of extrahepatic bile duct and gallbladder and Roux-en-Y hepaticojejunostomy were performed. On gross inspection, we could not find any tumor lesion in the resected specimen.However, the postoperative histopathologic examinations confirmed the presence of well-differentiated tubular adenocarcinoma with lymphovascular invasion. Most of the carcinoma remained within the mucosal layer, and the carcinoma was identified at both the distal and proximal surgical margins of the bile duct. We scheduled additional surgery to eradicate the residual carcinoma, but informed consent for the extra surgery could not be obtained.A close follow-up with abdominal computed tomography has been going on without either additional surgery or adjuvant chemotherapy about for 1 year. The patient has so far been clinically doing well without any obvious symptoms of recurrent disease. To our knowledge, this report is the youngest case of CC associated with CBD.     

胆总管急性梗阻时胆道压力胆管扩张黄疸三者的关系

目的 探讨胆总管急性梗阻时胆道压力、胆管扩张、黄疸三者的关系.方法 犬10条,在十二指肠上缘的胆总管内置入连接自动测压装置系统直经为3.5mm的小管,先测胆道基础压和胆总管直径,然后转开三通开关,通过直经3.5mm小管向胆总管内滴入美蓝盐水(NS 100 ml+美兰60 mg),20滴／min,等于造成胆道梗阻,按结果中显示的时间段测量胆道压力和胆总管直径,并观察美兰染色的胆汁返流至肝脏的情况.结果 滴人美兰盐水前,胆道压力为＜8 mm Hg(1 mm Hg =0.133 kPa),胆总管直径为4.0mm,肝脏色泽正常.经3.5 mm测压管向胆管内滴入美兰盐水7 min后,胆道压力上升至15.42 mm Hg,胆总管直径增宽至5.5 mm;15 min后,胆道压力上升至20mm Hg,胆总管直径6mm,肝脏开始变蓝、表示胆管内胆汁巳向肝内染色返流;30 min后,胆道压力上升至34 mm Hg,胆总管直径6.7mm,肝脏美蓝染色加深;45 min后,胆道压力21 mm Hg,胆总管直径7.5 mm,肝脏呈深蓝色;60 min后,胆道压力18 mm Hg,胆总管直径7.5 mm.结论 胆道压力升高后先有胆管扩张,后有黄疸;在一定范围内,胆管扩张程度与胆道压力呈正相关;黄疸程度与胆道压力和胆管扩张程度呈正相关.但胆道压力上升至34 mm Hg时不再升高,胆管扩张至7.5mm时不再扩张,表明胆道压力和胆管扩张均有一定的自限度.     

正常直径胆总管行腹腔镜下胆总管探查一期缝合的安全性及可行性

目的探讨正常直径胆总管行腹腔镜下胆总管探查一期缝合的安全性与可行性。方法回顾性分析盛京医院2014年1月1日至2019年11月30日收治的行腹腔镜下胆总管探查一期缝合的120例患者资料,其中男性44例,女性76例,平均年龄57.2(22.0~88.0)岁。根据胆总管直径大小将患者分为扩张组(>8 mm)和正常组(≤8 mm)。比较两组患者手术时间、术中出血量、术后住院时间、术后腹腔引流管拔除时间与术后并发症的发生情况。结果扩张组纳入76例患者,其中男性25例,女性51例,中位年龄62.5岁;正常组纳入44例,其中男性19例,女性25例,中位年龄57.5岁。两组患者性别、年龄、白蛋白水平、总胆红素等基线资料差异无统计学意义(P>0.05)。两组患者手术时间[正常组106.0(87.3,146.3)min比扩张组112.0(90.5,134.5)min]、术中出血量[正常组20(10,30)ml比扩张组20(10,20)ml]、术后住院时间[正常组7.0(5.3,9.0)d比扩张组7.0(5.0,7.0)d]、术后腹腔引流管拔除时间[正常组6(4,7)d比扩张组5(4,6)d]差异均无统计学意义(P>0.05)。两组患者术后胆漏、胆道狭窄、结石复发等并发症的发生率差异无统计学意义(P>0.05)。结论在正常直径(≤8 mm)胆总管的患者中行腹腔镜下胆总管探查联合一期缝合术安全有效。     

Dynamic study of pancreaticobiliary reflux using secretin-stimulated magnetic resonance cholangiopancreatography in patients with choledochal cysts

Background/Purpose

Refluxes through pancreaticobiliary maljunctions play an important role in the pathophysiology of choledochal cysts. Dynamic studies of the pancreaticobiliary tract were performed using secretin-stimulated magnetic resonance cholangiopancreatography.

Methods

Six patients with choledochal dilation were recruited for this study. Four patients exhibited cystic and 2 exhibited fusiform dilatation of the common bile duct (CBD). Magnetic resonance cholangiopancreatography images were obtained every minute during the 15-minute period after secretin stimulation. The sequential morphological changes in the biliary trees, pancreas, and duodenum were assessed, and the total pixel values of these organs were measured for each image, then plotted as a ratio against the baseline image.

Results

In 2 cases involving cystic dilatation, the intensity of bile duct images continued to rise after secretin stimulation. In a case involving fusiform dilatation, a transitory elevation in CBD intensity was observed. In 3 cases involving fusiform or cystic dilatation, the intensity of CBD did not change notably. In all cases, the duodenum was filled well after secretin stimulation.

Conclusions

The sustained elevation in bile duct intensity after secretin stimulation indicates reflux and bile stasis. Transitory elevation may indicate reflux without stasis. This method allows assessment of the dynamics of pancreatic and bile fluid under more physiologic condition.     

Primary yolk sac tumor of the common bile duct

Yolk sac tumor (YST) is a germ cell neoplasm usually arising from the gonads. Extragonadal YST has been reported in the mediastinum, retroperitoneum, liver, and pelvis. Primary YST of the common bile duct (CBD) has not been previously reported. We describe an 11-month-old girl with a YST arising from the CBD who presented with obstructive jaundice. Imaging studies revealed an intraluminal CBD mass with intrahepatic biliary dilatation and extraluminal infiltration of the head of the pancreas. A complete resection was carried out, and postoperative chemotherapy with cisplatin, etoposide and bleomycin was administered.     

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尽可能彻底切除异常扩张的胆管并实现胆汁与胰液完全分流是目前先天性胆管扩张症手术治疗的主要目标,但胆肠吻合的质量对远期疗效有重要影响,需严格遵循胆道外科基本原则实施操作。胆肠吻合口狭窄是先天性胆管扩张症术后远期疗效不佳的最常见原因。大致可分为两种类型,一类为原行囊肿空肠吻合或囊肿部分切除、上段残留囊肿空肠吻合者,胆肠吻合口相对于明显膨大的囊肿残腔呈"相对性狭窄";另一类为通常意义的胆肠吻合口狭窄,与囊肿上切缘位置过高、原有肝门区胆管结构异常、肝内胆管囊肿未妥善处理、吻合操作欠妥等有一定关系。总结此类病人术后胆肠吻合口狭窄及结石再生的原因,对更合理地治疗先天性胆管扩张症确有现实意义。     

Laparoscopic treatment for common bile duct stones by transcystic papilla balloon dilatation technique

The laparoscopic transcystic common bile duct (CBD) approach is becoming increasingly more refined as an ideal technique to deal with gallbladder stones (GBS) and common bile duct stones (CBDS) during a single operation. Our method, transcystic CBD exploration and papilla balloon dilatation (PBD), is an easier, safer, and less invasive technique than the transcystic approaches that have previously been reported. With our method, a sheath is introduced through the cystic duct into the CBD in order to allow catheter exchange, and the CBDS is flushed out through the papilla into the duodenum after PBD. We applied our new technique, without complication, to a patient with GBS and CBDS. Our technique is one of the safest, easiest, and least invasive methods for the treatment of patients with GBS and CBDS. Received: 22 July 1998/Accepted: 7 March 1999